# Treatment options for idiopathic pulmonary fibrosis (IPF)
In idiopathic pulmonary fibrosis, two medications are central to slowing disease progression. Additionally, supportive treatments are used to relieve symptoms and preserve lung function as much as possible. The approach is highly individual and depends on how advanced the disease is, how quickly it progresses, and how someone tolerates the medications.
Medicines against fibrosis
Nintedanib
ProveniIncluded in official guidelines, or approved by EMA or FDA
Nintedanib works by blocking certain groups of signals in body cells that lead to scarring in the lungs. This medication inhibits the activity of fibroblasts — the cells that produce excessive binding (collagen). Studies show that it can slow the decline of lung function.
Side effects that occur regularly include diarrhea (occurring in the majority of users), nausea, abdominal pain and decreased appetite. In some cases weight loss occurs. Recent research suggests that body composition may play a role in how someone responds to this medication, particularly for diarrhea. The medication can also affect bleeding tendency and wound healing.
Pirfenidon
ProveniIncluded in official guidelines, or approved by EMA or FDA
Pirfenidon works by slowing inflammation-related processes and fibrosis itself. It appears to act particularly on cells involved in scarring. This medication also demonstrably slows the decline of lung function.
Side effects include skin rash (occurring in a considerable proportion of patients, sometimes severe), nausea, diarrhea, fatigue and weight loss. Sensitivity to sunlight may increase. Liver function and kidney function values are regularly monitored because this medication can affect them.
Supportive treatments
Oxygen therapy
ProveniIncluded in official guidelines, or approved by EMA or FDA
In patients whose blood oxygen levels are too low (measured by a simple test), oxygen therapy is prescribed. This can take place at home (via concentrator or cylinders), during exertion or during the night, depending on when oxygen is low. Oxygen therapy relieves shortness of breath and protects the heart and brain from harmful effects of prolonged oxygen deprivation.
There are no serious side effects, but prolonged use of very dry oxygen can irritate airways; therefore oxygen is usually humidified.
Rehabilitation and movement
ProveniIncluded in official guidelines, or approved by EMA or FDA
Physiotherapy and guided physical exercise help maintain muscle strength and endurance, especially in the legs, chest and trunk. This can reduce shortness of breath on exertion and helps maintain someone's ability to perform daily tasks. Breathing exercises teach people to manage their breathing better.
There are no direct side effects, but exertion should be built up carefully to prevent exhaustion and overload.
Treatment of additional symptoms
ProveniIncluded in official guidelines, or approved by EMA or FDA
Pain, cough and insomnia are treated separately. For persistent cough, cough suppressants can be used. For anxiety and depression (which occur regularly with this chronic disease) psychological support or medications can help. This contributes to quality of life.
Under investigation
Nerandomilast
ResearchediPositive results in clinical studies, not yet standard treatment
Nerandomilast is an experimental agent that inhibits certain inflammatory pathways in the lungs. It is currently being investigated in clinical trials in patients with progressive fibrosis. To date, results have been promising regarding safety and tolerability, but definitive data on efficacy are still pending.
Therapies targeting specific cell mechanisms
ResearchediPositive results in clinical studies, not yet standard treatment
Research indicates that certain biological pathways — such as those involving protein HIF-1α and collagen-degrading proteins — can be influenced. These investigations are mainly taking place at the cellular level and are not yet considered standard treatment.
Stem cell therapies
ResearchediPositive results in clinical studies, not yet standard treatment
Specialized stem cells (such as those derived from human embryos) can theoretically break down collagen and send anti-inflammatory signals that inhibit fibrosis. This is being investigated in laboratory studies and early clinical trials, but is not yet available in routine care.
Selective interventions on gut microbiota
ResearchediPositive results in clinical studies, not yet standard treatment
New research suggests that the composition of gut bacteria is linked to outcomes in pulmonary fibrosis, including survival. This opens prospects for targeted dietary adjustments or specific bacterial colonies, but this approach is still in the research phase.
Inhaled medications in trial
Avalyn and ARO-MMP7
ExperimentaliOngoing in study setting, outcome still unknown
These agents are delivered directly into the lung (inhalation) and target key proteins in the fibrosis process. They are in early clinical testing. The potential advantage could be that they work directly in the affected tissue.
Treatments with limited evidence
Antiviral therapy
UnproveniNo scientific evidence that it works
There is suspicion that certain viruses play a role in the development of pulmonary fibrosis, but antiviral medications have so far provided no proven benefit in randomized studies.
Immunosuppressants (classical form)
Advised againstiProven ineffective or harmful, or dangerous in combination with your treatment
Previously, medications that suppress the immune system were sometimes given. This is now not recommended, because studies showed they can be harmful and provide no benefit.
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On progressive course and timing
Recent research emphasizes that treatment should preferably be started early, rather than waiting until the disease has already progressed significantly ('wait-to-fail'). This is because early intervention appears to produce better long-term results. Also being examined is the best appropriate order when two medications are needed simultaneously.
The choice of which medication is suitable usually depends on how well someone tolerates it, other conditions they have, and individual response to the medication. Doctor and patient discuss this choice step by step.
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_This information never replaces a doctor's judgment. Always discuss your situation with your own healthcare provider._